José Estevão-Costa

Delayed gastric emptying and gastroesophageal reflux: a pathophysiologic relationship.

Background Delayed gastric emptying (DGE) is frequent in patients with gastroesophageal reflux (GER), but its pathophysiologic role has not yet been established. To identify a relationship between DGE and GER, we assessed whether DGE increases esophageal acid exposure and the related importance of possible mechanisms. Methods Thirty pediatric patients with pathological GER were divided according to gastric emptying scintigraphy into a DGE group (n = 14) and normal-emptying group (n = 16). The esophageal pH-monitoring parameters of the two groups were compared with respect to the individual variation between postprandial and fasting periods. Results Patients with DGE had less total acid exposure than did those with normal emptying, but patients in both groups had a pathological fraction of time when pH was below 4 in both the postprandial (median: 18 vs. 27.6;P = 0.49) and fasting (8.5 vs. 23.9;P = 0.01) periods. Patients in the normal-emptying group had similar fraction of time when pH was below 4 in the postprandial and fasting periods. However, patients in the group with DGE had a fraction of time when pH was below 4 in the postprandial period that was almost double that presented in fasting period (postprandial to fasting ratio: 2.11:0.90;P = 0.002). The postprandial to fasting ratio for episodes per hour was similar in the two groups (1.81 vs. 1.79;P = 0.62). Patients with DGE had a significantly higher frequency of long episodes in the postprandial period than did those with normal emptying (62.5% vs. 38.2%;P = 0.04). The occurrence of the longest episode in the postprandial period was also significantly higher for patients with DGE (57.1% vs. 6.2%;P = 0.003). Conclusions DGE seems to accentuate postprandial reflux by increasing the volume of refluxate per episode of reflux through an underlying incompetent lower esophageal sphincter.

Gastric inflammatory myofibroblastic proliferation in children

Abstract Gastric inflammatory myofibroblastic proliferation (IMP) is an extremely rare entity in children, which to our knowledge has only been mentioned in case reports. We describe the ninth pediatric case and review the literature concerning the etiology, clinical and laboratory features, pathology, treatment, and outcome. There has been a predominance in preschool females. Abdominal pain, upper gastrointestinal hemorrhage, and an abdominal mass, either isolated or associated, have been the main clinical features. Iron-deficiency anemia has been a constant finding. Lesions are elevated and involve the full thickness of the gastric wall, usually with ulceration of the luminal surface; extragastric extension suggesting malignancy is frequent. Diagnosis is made by histology after surgical excision. There was no mortality directly related to gastric IMP, and only one case recurred after surgical excision. The pathogenesis is controversial, but the finding of Helicobacter pylori in our case may indicate an inflammatory origin. Awareness of this benign lesion and its mimicry of malignancy is important so that inappropriately aggressive therapy can be avoided.

Acute gastric volvulus secondary to a Morgagni hernia

Abstract Gastric volvulus (GV) is a rare surgical emergency in infancy and childhood. The first case of a child with an acute GV secondary to a Morgagni hernia (MH) is reported. The authors briefly discuss the etiology, clinical features, and management of acute GV and review the scattered reports of strangulated MHs in children.

Fetal heart development in the nitrofen-induced CDH rat model: The role of mechanical and nonmechanical factors

BACKGROUND/PURPOSE In congenital diaphragmatic hernia (CDH), it was recently shown that early and late gestational lung underdevelopment is caused by nonmechanical and mechanical factors, respectively. Heart underdevelopment, which might predict lung hypoplasia, is commonly attributed to mechanical factors. The authors analyzed whether nonmechanical and mechanical factors affect cardiac growth and correlations between lung and heart weights during gestation. METHODS Left-sided CDH was induced in pregnant Wistar rats by administration of nitrofen on E9.5. At selected gestational ages (E18, E20, and E22), the lungs and heart were harvested, weighed, and analyzed for DNA and protein contents. Left lung and heart weights were correlated at those gestational ages. Two experimental groups: nitrofen without CDH (nitrofen), and nitrofen with CDH (CDH), were compared with normal controls. RESULTS At E18, both nitrofen-exposed groups presented similar and significant left lung (LL) hypoplasia. As gestation progressed (E20 and E22), in the nitrofen group left lung (LL) hypoplasia decreased, whereas in the CDH group LL hypoplasia was exacerbated relative to normal controls. In contrast, at E18 and E20, heart-to-body weight ratios as well as cardiac DNA and protein contents were reduced significantly in all animals exposed to nitrofen, with no significant differences observed between nitrofen and CDH groups. As gestation progressed, the difference between cardiac parameters in nitrofen-exposed and normal control rats diminished, and at E22 no significant differences were documented. In the CDH group, significant correlations were seen between lung and heart weights at E18 (r = 0.65; P <.05) and E20 (r = 0.4; P <.05), whereas at term gestation (E22) no significant correlation was observed (r = 0.21, not significant). CONCLUSIONS Nonmechanical factors, which might be directed by nitrofen, play a role in the pathogenesis of lung and heart hypoplasia manifested precociously in fetal life, whereas mechanical compression might influence only lung growth during late gestation. Heart weight predicts lung weight only in early gestational ages.

Idiopathic perforation of the gallbladder: A novel differential diagnosis of acute abdomen

A six-year-old boy was referred with suspicion of acute appendicitis. He complained of vomiting and abdominal pain for the last 24 hours. Axillary temperature was 38oC and the abdomen was painful and tender. Complete blood count revealed leukocytosis. Abdominal ultrasonography demonstrated moderate free intraperitoneal fluid, mainly in the right iliac fossa and pelvis. An emergency laparotomy was performed with the presumptive diagnosis of peritonitis secondary to appendicitis but there was a 500 ml bile-stained ascites. Exploration of the abdominal cavity revealed a type I (3) oval perforation (2 cm) of the gallbladder infundibulum (Fig. 1). There were no gallstones or bile duct dilatation. Cholecystectomy was then performed and the child experienced an uneventful recovery. Histological examination did not reveal inflammatory infiltrate or any other significant alteration of the gallbladder wall. The cultures of peritoneal fluid were negative. Postoperative biliary MRI was normal. At 15-month follow-up the child continued to be well. DISCUSSION

Autotransplantation of the vermiform appendix for ureteral substitution

Ureteral substitution with vermiform appendix has been reported rarely. Herein is reported a case of a traumatic ureteropelvic disruption with a 7-cm long ureteral defect that was bridged successfully by appendix interposition for the first time in a child. The potential use of vermiform appendix to repair long ureteral defects provides an additional argument against incidental appendectomy.

Abdominal inflammatory myofibroblastic tumor a clinicopathologic study with reappraisal of biologic behavior.

BACKGROUND AND PURPOSE Inflammatory myofibroblastic tumor (IMT) is a proliferative lesion of controversial nosology and uncertain prognosis. In an attempt to acquire further understanding of pathogenesis and biologic behavior, we surveyed abdominal IMTs managed over the last 12 years at a single institution. METHODS Intra-abdominal IMTs treated between 1995 and 2007 were reviewed concerning demographic, clinical, and pathologic features as well as therapeutic management and outcome. All specimens were reevaluated by histologic examination and immunohistochemistry. RESULTS There were 7 patients (4 males; age range, 28 days to 14 years). Five lesions were located in alimentary tract: 1 gastric presenting with bleeding, 1 hepatic presenting with a thoracic wall mass, 1 pancreatic and 2 colonic presenting with obstructive symptoms. One splenic IMT was found incidentally. The remaining case arose from the adrenal gland and presented with a palpable mass. The gastric and adrenal IMTs had evidence of a previous or concomitant infectious setting. Five lesions were excised. The pancreatic IMT underwent a drainage procedure followed by steroid administration, and the hepatic lesion received antibiotics. Histopathology revealed characteristic findings of IMT. Expression of anaplastic lymphoma kinase was negative in all cases. At a median follow-up of 6 years (range, 3-15), all children were asymptomatic with no recurrences. The hepatic and pancreatic IMT displayed complete and near total regression, respectively. CONCLUSION A benign behavior of abdominal IMTs was observed even in patients not undergoing surgical excision. Although IMT remains a surgical disease, a conservative approach may be reasonable in select cases.

Minimal access surgery in the management of pediatric urolithiasis

PURPOSE In contrast to adult patients, a relatively large number of open surgical procedures are still needed in the treatment of urolithiasis in children. Since almost all open surgical techniques may be reproduced by minimal access surgery (MAS), there is a rationale to apply the latter in the management of pediatric urolithiasis. Our study aimed to assess the feasibility and outcome of MAS in the treatment of pediatric urinary calculi. MATERIALS AND METHODS The charts of patients with urolithiasis submitted to MAS between 1994 and 2007 were retrospectively reviewed. The inclusion criteria were contraindication for and failure of lithotripsy or endourology techniques. Demographic data, lithiasis characterization (location, dimension, composition), predisposing factors (anatomic or metabolic) and surgical approach (technique and outcome) were evaluated. RESULTS Fifteen consecutive patients (eight girls, seven boys) with a median age of 108 months (range: 10-297) were elected for MAS. Eleven (73%) children had associated urogenital malformations and three (20%) presented metabolic abnormalities. A total of 17 procedures were performed laparoscopically: three nephrolithotomies (one transperitoneal, two by retroperitoneoscopy), four pyelolithotomies (retro), three ureterolithotomies (trans) and seven cystolithotomies (suprapubic approach). Five patients underwent concomitant correction of urological anomalies (three calyceal diverticula, one obstructive megaureter, one ureteropelvic junction obstruction). Complete removal of calculi was accomplished in 14 (82%) procedures. There were two perioperative complications (one intraperitoneal vesical perforation and one perivesical urinoma). At a median follow up of 4 years (range: 1 month to 11 years), four patients have developed recurrence. CONCLUSIONS Minimal access surgery is an effective and safe approach for urolithiasis in children who are not candidates for minimally invasive modalities. Individualized application of different techniques may solve virtually all cases. In selected cases, the role of MAS as first-choice therapy deserves consideration.

Laparoscopic colonic mapping of dysganglionosis.

Abstract Retention of a proximal aganglionic segment or the unrecognized coexistence of other dysganglionoses may jeopardize the definitive surgical treatment of Hirschsprungs disease (HD). To assess the extent of the disease and/or the presence of other dysganglionoses without an additional laparotomy, we developed a laparoscopic-assisted technique to perform colonic full-thickness biopsies. After creation of a pneumoperitoneum, a 5-mm laparoscope is inserted in the supraumbilical area and a working 10/12-mm port is placed in the left iliac fossa. The sigmoid/descending colon is grasped and pulled through the abdominal wall and a full-thickness biopsy done. The same procedure is applied to the transverse and ascending colon. Rectal and colonic biopsy specimens were studied using enzyme histochemical methods. Over the last year, five children aged 7 months to 12 years with dysganglionosis underwent laparoscopic-assisted mapping of the colon. Previous rectal suction biopsies were diagnostic of HD in three patients and suspicious of hypoganglionosis in two. Proximal full-thickness biopsies revealed: normal colon in two cases of HD; coexistent type B intestinal neuronal dysplasia up to the descending colon in the other case of HD; and hypoganglionosis up to the ascending colon in the two patients with suspected hypoganglionosis. The procedures were performed easily, the patients being discharged after 36 to 48 h with no complications. Four children have already undergone pull-through procedures with resection of the affected colon in adhesion-free abdominal cavities and did not develop constipation or enterocolitis. Laparoscopic-assisted mapping of the entire colon is a simple, safe, and effective procedure that may contribute to improving the outcome of intestinal dysganglionosis by better characterization of the disease.

Minimally Invasive Repair of Morgagni Hernia - A Multicenter Case Series

Children may benefit from minimally invasive surgery (MIS) in the correction of Morgagni hernia (MH). The present study aims to evaluate the outcome of MIS through a multicenter study. National institutions that use MIS in the treatment of MH were included. Demographic, clinical and operative data were analyzed. Thirteen patients with MH (6 males) were operated using similar MIS technique (percutaneous stitches) at a mean age of 22.2±18.3 months. Six patients had chromosomopathies (46%), five with Down syndrome (39%). Respiratory complaints were the most common presentation (54%). Surgery lasted 95±23min. In none of the patients was the hernia sac removed; prosthesis was never used. In the immediate post-operative period, 4 patients (36%) were admitted to intensive care unit (all with Down syndrome); all patients started enteral feeds within the first 24h. With a mean follow-up of 56±16.6 months, there were two recurrences (18%) at the same institution, one of which was repaired with an absorbable suture; both with Down syndrome. The application of MIS in the MH repair is effective even in the presence of comorbidities such as Down syndrome; the latter influences the immediate postoperative recovery and possibly the recurrence rate. Removal of hernia sac does not seem necessary. Non-absorbable sutures may be more appropriate.